Background: Ureteral endometriosis commonly presents with non-specific symptoms or no symptoms. Diagnostic challenges are multifactorial but essentially caused by diagnostic delay due to the silent nature of the disease. Management requires a multidisciplinary approach to optimise the outcome. Case Report: A 29-year-old woman presented with severe deep infiltrating endometriosis resulting in bilateral hydronephrosis and loss of left kidney function. Laparoscopic excision of deep infiltrating endometriosis, ureterolysis, bowel resection and colostomy formation were performed. Eventually the left non-functional kidney required nephrectomy. Conclusion: The presence of posterior deep infiltrating endometriosis with uterosacral involvement should increase clinical suspicion of ureteral endometriosis and trigger targeted investigations. The best outcome is achieved by the individualisation of patient’s care.